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Diffuse midline glioma H3K27-altered with thoracic epidural metastasis: illustrative case
Kan Sumita1, Masahiro Sawada1, Etsuko Yamamoto Hattori1
1Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
Background:
Diffuse midline glioma (DMG) H3K27-altered is a newly recognized diffuse high-grade tumor entity in the 5th edition of the WHO classification of CNS tumors. Spinal extradural metastasis is extremely rare in patients with DMG H3K27-altered, while the occurrence of spinal intramedullary DMG, intradural dissemination, and osseous metastasis has been reported.
Observations:
The authors report the case of a 6-year-old female presenting with acute-onset urinary retention and paraplegia, who was found to have a pontine DMG, an intradural mass at the level of T1-6, and an extradural lesion at T6-9. The thecal sac was severely compressed by the extradural mass; thus, a T6-9 laminectomy and extradural tumor resection were performed. After the procedure, an amelioration of weakness was observed. The patient was treated with radiotherapy to the spine. Pathology revealed an increase in chromatin, the proliferation of atypical cells, and microvascular proliferation. The H3K27 mutation was confirmed.
Lessons:
This case demonstrates an operative view in the metastasis of DMG H3K27-altered to the spinal epidural space without visceral or osseous metastasis. The possibility of spinal epidural metastasis and its surgical treatability should be considered if patients with DMG present spinal cord symptoms. https://thejns.org/doi/10.3171/CASE25249.
Insights
Spinal epidural metastasis from diffuse midline glioma (DMG) H3K27-altered is rare but surgically treatable. Early consideration is vital for patients with DMG presenting with spinal cord symptoms.
Area of Science:
- Neuro-oncology
- Spinal Surgery
- Pathology
Background:
- Diffuse midline glioma (DMG) H3K27-altered is a newly classified high-grade CNS tumor.
- Spinal extradural metastasis is an exceptionally rare presentation of DMG H3K27-altered.
Purpose of the Study:
- To report a rare case of spinal extradural metastasis in a pediatric patient with DMG H3K27-altered.
- To highlight the surgical treatability and clinical considerations for spinal epidural metastasis in DMG.
Main Methods:
- Case report of a 6-year-old female with pontine DMG presenting with paraplegia and urinary retention.
- Surgical resection of T6-9 extradural mass with laminectomy.
- Postoperative radiotherapy and pathological confirmation of H3K27 mutation.
Main Results:
- The patient presented with a pontine DMG, intradural T1-6 mass, and T6-9 extradural mass causing severe thecal sac compression.
- Surgical decompression led to improved motor function.
- Histopathology confirmed DMG with H3K27 mutation.
Conclusions:
- This case illustrates surgical management of spinal epidural metastasis from DMG H3K27-altered.
- Spinal epidural metastasis should be considered in DMG patients with new-onset spinal symptoms.
- Surgical intervention can be beneficial for spinal cord compression caused by extradural DMG metastasis.
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